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 Fourth
Edition

Atlas of
UNCOMMON
PAIN SYNDROMES
Steven D. Waldman, MD, JD
Vice Dean
Chairman and Professor
Department of Humanities and Bioethics
University of Missouri—Kansas City
Kansas City, Missouri
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899

ATLAS OF UNCOMMON PAIN SYNDROMES, FOURTH EDITION ISBN: 978-0-323-64077-0

Copyright © 2020 by Elsevier Inc. All rights reserved.

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 This book is dedicated to David Mayo
My little boy yesterday, my friend today, my son forever.
SDW
2019

WHAT OCCAM, KISS, ZEBRAS, AND MICKEY
GILLEY ALL HAVE IN COMMON
It has been said that the three most dangerous things in medicine
are: (1) a medical student with a sharp object; (2) a resident armed
with a recently published study from NEJM, and; (3) an attending
physician with an anecdote. One must suspect that #2 was at play
when in the 1940’s, while on rounds at the University of Mary-
land Hospital in Baltimore, Maryland, Theodore Woodward, MD,
stated that “If you hear hoof beats out on Green Street, don’t look for
zebras”! How this admonition to aspiring physicians morphed into
“when you hear hoof beats, look for horses, not zebras” is anybody’s
guess. (My son who was an ophthalmology resident in Baltimore
suggests that this sage piece of advice was most likely accompa-
nied by a long-winded and confusing anecdote—see #3 above).
On the surface, most of us would agree with Dr. Woodward’s
logic that the most common things are the most common. Occam
agreed, when in the 14th century he put forth the philosophical
tenant of parsimony that proposed that simpler explanations are, all
things being equal, almost always better than more complex ones.
He used a razor to “shave away” unnecessary or extraneous data
to get to the simplest solution. When you think about it, a razor
was all the rage as a medical instrument in the 14th century, so it is
not surprising that Occam chose it as his preferred medical device.
Occam’s razor certainly has a nice ring to it—better than Occam’s
MRI—which would no doubt be the name of his maxim if he had
lived in the 21st century, given that the MRI is certainly our most
current popular medical device for “shaving away” extraneous data.
Which brings us to KISS. Not the Gene Simmons’ rock band
KISS, but the admonition “Keep It Simple Stupid.” KISS was set
forth by Lockhead aeronautical engineer Kelly Johnson when he
handed his design team a few simple tools and challenged them
to design military jets that could be easily fixed with the simple
tools that were available in combat situations. It is still not exactly
clear to me who was “stupid,” but I certainly hope it is not the guys
who fix the jets I fly on. KISS makes sense when designing jet
engines, but one has to ask what KISS has to do with the individual
patient—the sick one—the scared one—the one you worry about
in the middle of the night. Unfortunately, very little. Because for
the individual patient with a difficult diagnosis, it turns out that a
guy named Harry Hickam was probably more correct than Occam.
While on teaching rounds at Duke University, Harry
Hickam, MD, admonished his students and residents that
“patients can have as many diseases as they damn well please”!
(See also #3 above.) He correctly posited that when diagnosing
the individual patient, using Occam’s razor often provides the
correct diagnosis. But more often than we would care to admit,
when dealing with a patient presenting with a perplexing con-
stellation of signs and symptoms, it can just as easily provide the
wrong one. In fact, overreliance on Occam’s razor can be down-
right dangerous for both the patient and physician.
Often the simplest or, in the case of medical diagnosis, the
most common illness is exactly what is causing the patient’s
P R E FA C E
symptoms. But sometimes, in our almost obsessive desire to
make the diagnosis, simplicity is our enemy. In our haste to
make the patient fit the diagnosis, we get it wrong. Uncommon
diseases are called uncommon diseases because they are uncom-
mon—they are not called unknown diseases (see below). Since
the beginning of time healers have recognized that the correct
diagnosis is the key to getting the patient well, and as a corol-
lary, they also realized that the wrong diagnosis is not a “practice
builder.” Which brings us to country music legend Mickey Gilley.
In 1976 Mickey Gilley recorded the classic country ballad “The
Girl’s All Get Prettier At Closing Time. (FYI, 12 straight weeks
charted at #1). This song is a plaintive lament about loneliness and
late-night desperation. It explores how one’s perception of things
can change as circumstances change. What turns an unknown and
undiagnosable disease into an uncommon disease is knowledge.
What changes our perception of what a constellation of symp-
toms and physical findings mean when we are confronted with
a sick patient with an elusive diagnosis is knowledge. As we gain
more clinical experience, things that were once unknown become
known—even commonplace. The more we hone our clinical
acumen, the easier it is to put the pieces together—the jumble of
disparate signs and symptoms come into focus—and then all of a
sudden, we have a diagnosis—a diagnosis we will never miss again!
The Atlas of Uncommon Pain Syndromes, Fourth Edition,
seeks to accomplish three things: The first is to familiarize the
clinician with a group of uncommon pain syndromes that occur
with enough frequency that they merit serious study—not rare
or orphan diseases—just uncommon ones that are often mis-
diagnosed. Second, this text is written with the goal of helping
the clinician reinforce his or her knowledge of common pain
syndromes to help in those situations when Occam is relatively
correct—when the pieces of the puzzle don’t quite fit the simple
diagnosis. The third goal is more about the clinician and a little
less about the patient. It is about what attracted many of us to
medicine to begin with. It is the irresistible charm of being pre-
sented with a difficult clinical problem and getting it right. What
a great feeling that is! I hope you enjoy reading Atlas of Uncom-
mon Pain Syndromes, Fourth Edition, as much as I did writing it.
Steven D. Waldman, MD, JD
PS: The following ad recently ran in a local Kansas newspaper.
Perhaps the hoof beats were zebras after all!
TAMED YOUNG MALE AND FEMALE ZEBRAS FOR
SALE
($2,000.00)
I have available young Male and Female zebra babies for sale to good
and lovely homes who knows about the tamed zebras, feel free to send
us emails for more details and pictures, please only serious inquiries.
Category: Pets »Horses
Ad ID: 1564632
Date: May 16, 2018
vii
 SECTION 1 Headache and Facial Pain Syndromes

1
Ice Pick Headache

ICD-10 CODE R51 TESTING

Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps to identify abnormalities that
CLINICAL SYNDROME may put the patient at risk for neurological disasters secondary
Ice pick headache is a constellation of symptoms consisting of to intracranial and brainstem pathological conditions, includ-
paroxysms of stabbing jabs and jolts that occur primarily in the ing tumors and demyelinating disease (Fig. 1.2). Magnetic
first division of the trigeminal nerve. These paroxysms of pain resonance angiography (MRA) also may be useful in helping
may occur as a single jab or a series of jabs that lasts for a frac- identify aneurysms, which may be responsible for the patient’s
tion of a second followed by relatively pain-free episodes. The neurological findings. In patients who cannot undergo MRI,
pain of ice pick headache occurs in irregular intervals of hours such as a patient with a pacemaker, computed tomography (CT)
to days. Similar to cluster headache, ice pick headache is an epi- is a reasonable second choice. Radionuclide bone scanning and
sodic disorder that is characterized by clusters of painful attacks plain radiography are indicated if fracture or bony abnormality,
followed by pain-free periods. Episodes of ice pick headache
usually occur on the same side, but in rare patients the pain may
move to the same anatomical region on the contralateral side.
Ice pick headache occurs more commonly in women and is gen-
erally not seen before the fourth decade of life, but rare reports
of children suffering from ice pick headache sporadically appear
in the literature. Synonyms for ice pick headache include jabs
and jolts headache, primary stabbing headache, ophthalmody-
nia periodica, and idiopathic stabbing headache.

SIGNS AND SYMPTOMS

A patient suffering from ice pick headache complains of jolts
or jabs of pain in the orbit, temple, or parietal region (Fig. 1.1).
Some patients describe the pain of ice pick headache as a sud-
den smack or slap on the side of the head. Similar to patients
suffering from trigeminal neuralgia, a patient suffering from ice
pick headache may exhibit involuntary muscle spasms of the
affected area in response to the paroxysms of pain. In contrast to
trigeminal neuralgia, involving the first division of the trigem-
inal nerve, there are no trigger areas that induce the pain of ice
pick headache. The neurological examination of a patient suf-
fering from ice pick headache is normal. Some patients exhibit
anxiety and depression because the intensity of pain associated
with ice pick headache leads many patients to believe they have Fig. 1.1 Ice pick headache is characterized by jabs or jolts in the orbit,
a brain tumor. temple, or parietal region.

1
2 SECTION 1 Headache and Facial Pain Syndromes
Fig. 1.2 Diffuse Pachymeningeal and Calvarial Metastasis From
Carcinoma of the Breast Axial T1-weighted postgadolinium MRI
shows diffuse nodular and bandlike contrast-enhanced thickening of the
dura over the high right frontoparietal convexity (arrow). (From Haaga
JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole
Body. 4th ed. Philadelphia: Mosby; 2003:198.)
such as metastatic disease, is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry should be performed if the diagnosis of ice pick head-
ache is in question. Intraocular pressure should be measured if
glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Ice pick headache is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic ice pick
headache include trigeminal neuralgia involving the first divi-
sion of the trigeminal nerve, demyelinating disease, and chronic
paroxysmal hemicrania. Trigeminal neuralgia involving the first
division of the trigeminal nerve is uncommon and is charac-
terized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological findings,
including optic neuritis and other motor and sensory abnor-
malities. The pain of chronic paroxysmal hemicrania lasts much
longer than the pain of ice pick headache and is associated with
redness and watering of the ipsilateral eye.
TREATMENT
Ice pick headache uniformly responds to treatment with indo-
methacin. Failure to respond to indomethacin puts the diag-
nosis of ice pick headache in question. A starting dosage of 25
mg daily for 2 days and titrating to 25 mg three times per day
is a reasonable treatment approach. This dose may be carefully
increased to 150 mg per day. Indomethacin must be used care-
fully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to
cyclooxygenase-2 (COX-2) inhibitors in the treatment of ice
pick headache have been noted in the headache literature.
Underlying sleep disturbance and depression are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to correctly diagnose ice pick headache may put the
patient at risk if intracranial pathological conditions or demy-
elinating disease, which may mimic the clinical presentation of
chronic paroxysmal hemicrania, is overlooked. MRI is indicated
in all patients thought to be suffering from ice pick headache.
Failure to diagnose glaucoma, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.
CLINICAL PEARLS The diagnosis of ice pick headache is made by
obtaining a thorough, targeted headache history. Patients suffering from ice
pick headache should have a normal neurological examination. If the results of
the neurological examination are abnormal, the diagnosis of ice pick headache
should be discarded and a careful search for the cause of the neurological
findings should be undertaken.
SUGGESTED READINGS
Cutrer FM, Boes CJ. Cough, exertional, and sex headaches. Neurol
Clin. 2004;22:133–149.
Dafer RM. Neurostimulation in headache disorders. Neurol Clin.
2010;28:835–841.
Loulwah O, Jan MMS. Primary stabbing “ice-pick” headache. Pediat-
ric Neurology. 2011;45(4):268.
Matthew NT. Indomethacin responsive headache syndromes: head-
ache. J Head Face Pain. 1981;21:147–150.
Pascual J. Other primary headaches. Neurol Clin. 2009;27:557–571.
Rampello L, Malaguarnera M, Rampello L, et al. Stabbing headache
in patients with autoimmune disorders. Clin Neurol Neurosurg.
2012;114(6):751–753.
Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exer-
tional and sexual headaches in a Turkish population of headache
patients. J Clin Neurosci. 2008;15:774–777.

ICD-10 CODE G50.0
CLINICAL SYNDROME
The pain of supraorbital neuralgia is characterized as persistent
pain in the supraorbital region and forehead with occasional
sudden, shock-like paresthesias in the distribution of the supra-
orbital nerves. Sinus headache involving the frontal sinuses,
which is much more common than supraorbital neuralgia, can
mimic the pain of supraorbital neuralgia. Supraorbital neuralgia
is the result of compression or trauma of the supraorbital nerves
as the nerves exit the supraorbital foramen. Such trauma can be
in the form of blunt trauma directly to the nerve, such as when
the forehead hits the steering wheel during a motor vehicle acci-
dent, or repetitive microtrauma resulting from wearing welding
or swim goggles that are too tight. This clinical syndrome also is
known as swimmer’s headache.
SIGNS AND SYMPTOMS
The supraorbital nerve arises from fibers of the frontal nerve,
which is the largest branch of the ophthalmic nerve. The fron-
tal nerve enters the orbit via the superior orbital fissure and
passes anteriorly beneath the periosteum of the roof of the
orbit. The frontal nerve gives off a larger lateral branch, the
supraorbital nerve, and a smaller medial branch, the supra-
trochlear nerve. Both exit the orbit anteriorly. The supraorbital
nerve sends fibers all the way to the vertex of the scalp and
provides sensory innervation to the forehead, upper eyelid,
and anterior scalp (Fig. 2.1). The pain of supraorbital neuralgia
is characterized as persistent pain in the supraorbital region
and forehead with occasional sudden, shock-like paresthesias
in the distribution of the supraorbital nerves. Occasionally, a
patient suffering from supraorbital neuralgia complains that
the hair on the front of the head hurts (Fig. 2.2). Supraorbital
nerve block is useful in the diagnosis and treatment of supra-
orbital neuralgia.
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
2
Supraorbital Neuralgia
may put the patient at risk for neurological disasters secondary
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 2.3). Magnetic
resonance angiography (MRA) also may be useful in helping
identify aneurysms, which may be responsible for the patient’s
neurological findings. In patients who cannot undergo MRI,
such as a patient with a pacemaker, computed tomography (CT)
is a reasonable second choice. Radionuclide bone scan, CT, and
plain radiography are indicated if sinus disease, fracture, or
bony abnormality such as metastatic disease is considered in
the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of supra-
orbital neuralgia is in question. Intraocular pressure should be
measured if glaucoma is suspected (Fig. 2.4).
DIFFERENTIAL DIAGNOSIS
Supraorbital neuralgia is a clinical diagnosis supported by a
combination of clinical history, normal physical examination,
radiography, CT, and MRI. Pain syndromes that may mimic
supraorbital neuralgia include ice pick headache, trigeminal
neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania.
Trigeminal neuralgia involving the first division of the trigemi-
nal nerve is uncommon and is characterized by trigger areas and
tic-like movements. Demyelinating disease is generally associ-
ated with other neurological findings, including optic neuritis
and other motor and sensory abnormalities. The pain of chronic
paroxysmal hemicrania lasts much longer than the paroxysmal
pain of supraorbital neuralgia and is associated with redness
and watering of the ipsilateral eye.
TREATMENT
The primary treatment intervention for supraorbital neuralgia is
the identification and removal of anything causing compression
of the supraorbital nerves (e.g., tight welding or swim goggles).
A brief trial of simple analgesics alone or in combination with
gabapentin also should be considered. For patients who do not
respond to these treatments, supraorbital nerve block with local
anesthetic and a steroid is a reasonable next step. Ultrasound
guidance for needle placement may be useful when performing
supraorbital nerve block.
3
4 SECTION 1 Headache and Facial Pain Syndromes
Inflamed
supraorbital n.
Fig. 2.1 The supraorbital nerve sends fibers all the way to the vertex
of the scalp and provides sensory innervation to the forehead, upper
eyelid, and anterior scalp. n., Nerve.
Fig. 2.2 Occasionally a patient with supraorbital neuralgia complains
that the hair on the front of the head hurts. The supraorbital nerve sends
fibers all the way to the vertex of the scalp and provides sensory inner-
vation to the forehead, upper eyelid, and anterior scalp.
To perform supraorbital nerve block, the patient is placed in
the supine position. Using a 10-mL sterile syringe, 3 mL of local
anesthetic is drawn up. When treating supraorbital neuralgia
with supraorbital nerve block, 80 mg of depot steroid is added
to the local anesthetic with the first block, and 40 mg of depot
steroid is added with subsequent blocks.
The supraorbital notch on the affected side is identified by
palpation. The skin overlying the notch is prepared with anti-
septic solution, with care taken to avoid spillage into the eye. A
25-gauge, 1½-inch needle is inserted at the level of the supraor-
bital notch and is advanced medially approximately 15 degrees
off the perpendicular to avoid entering the foramen. The needle
is advanced until it approaches the periosteum of the under-
lying bone (Fig. 2.5). A paresthesia may be elicited, and the
patient should be warned of such. The needle should not enter
the supraorbital foramen; if this occurs, the needle should be
withdrawn and redirected slightly more medially.
Because of the loose alveolar tissue of the eyelid, a gauze
sponge should be used to apply gentle pressure on the upper
eyelid and supraorbital tissues before injection of solution to
prevent the injectate from dissecting inferiorly into these tis-
sues. This pressure should be maintained after the procedure to
avoid periorbital hematoma and ecchymosis.
After gentle aspiration, 3 mL of solution is injected in a fan-
like distribution. If blockade of the supratrochlear nerve also
is desired, the needle is redirected medially and, after careful
aspiration, an additional 3 mL of solution is injected in a fanlike
manner. In rare cases, destruction of the supraorbital nerve by
radiofrequency lesioning or supraorbital nerve stimulation may
be required to provide long-lasting relief (Fig. 2.6).
Underlying sleep disturbance and depression associated
with the pain of supraorbital neuralgia are best treated with a
tricyclic antidepressant compound, such as nortriptyline. The
tricyclic antidepressant can be started at a single bedtime dose
of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose supraorbital neuralgia correctly may put
the patient at risk if an intracranial pathological condition or
demyelinating disease, which may mimic the clinical presenta-
tion of supraorbital neuralgia, is overlooked. MRI is indicated
in all patients thought to have supraorbital neuralgia. Failure to
diagnose glaucoma, which also may cause intermittent ocular
pain, may result in permanent loss of sight.
The forehead and scalp are highly vascular, and when per-
forming supraorbital nerve block the clinician should carefully
calculate the total milligram dosage of local anesthetic that may
be given safely, especially if bilateral nerve blocks are being per-
formed. This vascularity gives rise to an increased incidence of
postblock ecchymosis and hematoma formation. Despite the
vascularity of this anatomical region, this technique can be per-
formed safely in the presence of anticoagulation by using a 25-
or 27-gauge needle, albeit at increased risk for hematoma, if the
clinical situation dictates a favorable risk-to-benefit ratio. These
complications can be decreased if manual pressure is applied to
 CHAPTER 2 Supraorbital Neuralgia 5

A B

C
Fig. 2.3 Subdural Empyema in a Patient With Sinusitis (A) T2-weighted MRI shows high-signal-intensity
extraaxial fluid collection in the right frontal convexity and along the falx on the right side. (B and C) Gado-
linium-enhanced MRI shows extraaxial fluid collections in the right frontal convexity and along the falx with
intense peripheral enhancement. The signal intensity of the fluid collection is slightly higher than that of
cerebrospinal fluid. (From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body.
4th ed. Philadelphia: Mosby; 2003:209.)
6 SECTION 1 Headache and Facial Pain Syndromes

Fixed
semidilated
Hazy corneal reflex pupil
signifying edema
Cataractous
lens

Opaque thickened
edematous cornea
Cataractous lens
Shallow anterior
chamber

Fig. 2.4 Acute Angle Closure Resulting From an Intumescent Cataractous Lens The eye is red with a
hazy view of the anterior segment from corneal edema, with a fixed, irregular, semidilated pupil from iris
infarction. The slit image shows the corneal edema and a very shallow anterior chamber. Some uveitis may be
present because of ischemia, and this must be differentiated from the larger accumulations of lens material
and macrophages seen with phacolytic glaucoma. (From Spalton DJ, Hitchings RA, Hunter P. Atlas of Clinical
Ophthalmology. 3rd ed. London: Mosby; 2005:225.)

Supraorbital n.

Supraorbital notch

Fig. 2.6 A three-dimensional CT reconstruction, taken prior to the pro-

cedure, showing the position of the supraorbital foramen (arrow). (From
Huibin Q, Jianxing L, Guangyu H, et al. The treatment of first division
Fig. 2.5 Injection Technique for Relieving the Pain of Supraorbital
idiopathic trigeminal neuralgia with radiofrequency thermocoagulation
Neuralgia n., Nerve. (From Waldman SD. Atlas of Pain Management
of the peripheral branches compared to conventional radiofrequency.
Injection Techniques. 2nd ed. Philadelphia: Saunders; 2007.)
J Clin Neurosci. 2009;16(11):1425–1429. ISSN 0967-5868, https://doi.
org/10.1016/j.jocn.2009.01.021.)

the area of the block immediately after injection. Application of
cold packs for 20-minute periods after the block also decreases
the amount of postprocedure pain and bleeding.
CLINICAL PEARLS Supraorbital nerve block is especially useful in the
diagnosis and palliation of pain secondary to supraorbital neuralgia. The first
step in the management of this unusual cause of headache is the correct fit-
ting of swimming goggles that do not compress the supraorbital nerves. Coex-
istent frontal sinusitis should be ruled out in patients who do not respond
rapidly to a change in swim goggles and a series of the previously mentioned
nerve blocks. Any patient with headaches severe enough to require neural
blockade as part of the treatment plan should undergo MRI of the head to rule
out unsuspected intracranial pathological conditions.
CHAPTER 2 Supraorbital Neuralgia 7
SUGGESTED READINGS
Hillerup S, Jensen RH, Ersbøll BK. Trigeminal nerve injury associated
with injection of local anesthetics: needle lesion or nneurotoxicity?
J Am Dental Assoc. 2011;142(5):531–539.
Levin M. Nerve blocks and nerve stimulation in headache disorders.
Tech Reg Anesth Pain Manage. 2009;13:42–49.
Levin M. Nerve blocks in the treatment of headache. Neurotherapeu-
tics. 2010;7:197–203.
Waldman SD. Swimmer’s headache. In: Waldman SD, ed. Atlas of
Pain Management Injection Techniques. Philadelphia: Saunders;
2007:7–10.
Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
view. Philadelphia: Saunders; 2009:15–17.
 3
Chronic Paroxysmal Hemicrania

may put the patient at risk for neurological disasters secondary

ICD-10 CODE R51
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 3.2). Magnetic res-
onance angiography (MRA) also may be useful in identifying
aneurysms, which may be responsible for the patient’s neuro-
CLINICAL SYNDROME logical findings. In patients who cannot undergo MRI, such
Chronic paroxysmal hemicrania, which is also known as as a patient with a pacemaker, computed tomography (CT) is
Sjaastad syndrome, shares many characteristics of its more a reasonable second choice. Radionuclide bone scanning and
common analogue, cluster headache, but has several important
differences (Table 3.1). Similar to cluster headache, chronic par-
oxysmal hemicrania is a severe, episodic, unilateral headache TABLE 3.1 Comparison of Cluster Headache
that affects the periorbital and retroorbital regions. In contrast and Chronic Paroxysmal Hemicrania
to cluster headache, which occurs 10 times more commonly
Chronic
in men, chronic paroxysmal hemicrania occurs primarily in
Paroxysmal
women (Fig. 3.1). The duration of pain associated with chronic
Comparison Factors Cluster Headache Hemicrania
paroxysmal hemicrania is shorter than that of cluster headache,
Gender predominance Male Female
lasting 5 to 45 minutes. This pain does not follow the chronobi-
Response to indomethacin Negative Positive
ological pattern seen in patients with cluster headache. Patients
Chronobiological pattern Positive Negative
with chronic paroxysmal hemicrania usually experience more Alcohol trigger Positive Negative
than five attacks per day. Chronic paroxysmal hemicrania uni- Length of attacks Longer Shorter
formly responds to indomethacin, whereas cluster headache Horner syndrome Present Present
does not.

SIGNS AND SYMPTOMS

During attacks of chronic paroxysmal hemicrania, patients
exhibit the following physical findings suggestive of Horner
syndrome on the ipsilateral side of the pain:
• Conjunctival and scleral injection
• Lacrimation
• Nasal congestion
• Rhinorrhea
• Ptosis of the eyelid
As in cluster headache, the patient may become agitated during
attacks, rather than seeking dark and quiet as does the patient
with migraine. In contrast to cluster headache, alcohol consump-
tion does not seem to trigger attacks of chronic paroxysmal hemi-
crania. Between attacks, the neurological examination of a patient
with chronic paroxysmal hemicrania should be normal.

TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents. Fig. 3.1 In contrast to cluster headache, which occurs primarily in men,
MRI is highly accurate and helps to identify abnormalities that chronic paroxysmal hemicrania occurs primarily in women.

8
 CHAPTER 3 Chronic Paroxysmal Hemicrania 9

plain radiography are indicated if fracture or bony abnormal-

ity such as metastatic disease is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
chronic paroxysmal hemicrania is in question. Intraocular pres-
sure should be measured if glaucoma is suspected.

DIFFERENTIAL DIAGNOSIS
Chronic paroxysmal hemicrania is a clinical diagnosis sup-
ported by a combination of clinical history, abnormal physi-
cal examination during attacks, radiography, and MRI. Pain
syndromes that may mimic chronic paroxysmal hemicrania
include cluster headache, trigeminal neuralgia involving the
A
first division of the trigeminal nerve, demyelinating disease,
and ice pick headache. Trigeminal neuralgia involving the first
division of the trigeminal nerve is uncommon and is charac-
terized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological find-
ings, including optic neuritis and other motor and sensory
abnormalities. The pain of cluster headache lasts much longer
than the pain of chronic paroxysmal hemicrania, and cluster
headache has a male predominance, a chronobiological pat-
tern of attacks, and a lack of response to treatment with indo-
methacin (Fig. 3.3).

B oxygenase-2 (COX-2) inhibitors in the treatment of chronic
Fig. 3.2 Sagittal (A) and semiaxial (B) T2-weighted images of a massive
prolactinoma in a 41-year-old man with chronic daily headache. (From
Benitez-Rosario MA, McDarby G, Doyle R, et al. Chronic cluster-like
headache secondary to prolactinoma: uncommon cephalalgia in asso-
ciation with brain tumors. J Pain Symptom Manage. 2009;37:271–276.)
TREATMENT
Chronic paroxysmal hemicrania uniformly responds to
treatment with indomethacin. Failure to respond to indo-
methacin puts the diagnosis of chronic paroxysmal hemicra-
nia in question. A starting dose of 25 mg daily for 2 days
and titrating to 25 mg three times per day is a reasonable
treatment approach. This dose may be carefully increased up
to 150 mg per day. Indomethacin must be used carefully, if
at all, in patients with peptic ulcer disease or impaired renal
function. Anecdotal reports of a positive response to cyclo-
paroxysmal hemicrania have been noted in the headache
literature. Underlying sleep disturbance and depression are
best treated with a tricyclic antidepressant compound, such
as nortriptyline, which can be started at a single bedtime
dose of 25 mg.

SUNCT Paroxysmal hemicrania Cluster headache

5 s-4 min 2-30 min 15-180 min Time

Overlap between duration Overlap between duration

Fig. 3.3 Overlap between attack duration in trigeminal autonomic cephalalgias. The duration of each trigeminal
autonomic cephalalgia is specified by the International Classification of Headache Disorders. (From Silber-
stein SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am.
2013;97(2):321–328.)
10 SECTION 1 Headache and Facial Pain Syndromes

TABLE 3.2 Characteristics of the Trigeminal SUGGESTED READINGS

Autonomic Cephalgias Benitez-Rosario MA, McDarby G, Doyle R, Fabby C. Chronic ­cluster-
• Unilateral like headache secondary to prolactinoma: uncommon cephalalgia in
• Short duration association with brain tumors. J Pain Symptom Manage. 2009;37:
• High frequency 271–276.
• Orbital, periorbital, or temporal
Benoliel R, Sharav Y. Paroxysmal hemicrania: case studies and review
• Associated autonomic symptoms
of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol
• Lacrimation
Endodontol. 1998;85:285–292.
• Conjunctival injection
Camarda C, Camarda R, Monastero R. Chronic paroxysmal hemicra-
• Nasal congestion
• Ptosis nia and hemicrania continua responding to topiramate: two case
• Eyelid edema reports. Clin Neurol Neurosurg. 2008;110:88–91.
Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalalgias.
II. Paroxysmal hemicrania. Oral Surg Oral Med Oral Pathol Oral
Radiol Endodontol. 2007;104:640–646.
COMPLICATIONS AND PITFALLS Schytz HW, Hargreaves R, Ashina M. Challenges in developing drugs
for primary headaches. Prog Neurobiol. 2017;152:70–88.
Failure to diagnose chronic paroxysmal hemicrania correctly
Sjaastad O. Chronic paroxysmal hemicrania: clinical aspects and con-
may put the patient at risk if intracranial pathological condi- troversies. In: Blau JN, ed. Migraine: Clinical, Therapeutic, Conceptu-
tions or demyelinating disease that may mimic the clinical pre- al and Research Aspects. London: Chapman & Hall; 1987:135–152.
sentation of chronic paroxysmal hemicrania is overlooked. MRI Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias
is indicated in all patients thought to have chronic paroxysmal other than cluster headache. Med Clin North Am. 2013;97(2):
hemicrania. Failure to diagnose glaucoma, which may cause 321–328.
intermittent ocular pain, may result in permanent loss of sight. Talvik I, Koch K, Kolk A, Talvik T. Chronic paroxysmal hemicrania in
a 3-year, 10-month-old female. Pediatr Neurol. 2006;34:225–227.
CLINICAL PEARLS Chronic paroxysmal hemicrania is classified as a
trigeminal autonomic cephalgia. The trigeminal autonomic cephalgias are a
group of distinct headache syndromes that share a number of common and
often overlapping clinical characteristics (Table 3.2). The diagnosis of chronic
paroxysmal hemicrania is made by obtaining a thorough, targeted headache
history. Between attacks, patients with chronic paroxysmal hemicrania should
have a normal neurological examination. If the neurological examination is
abnormal between attacks, the diagnosis of chronic paroxysmal hemicrania
should be discarded and a careful search for the cause of the patient’s neuro-
logical findings should be undertaken.
 4
Hemicrania Continua

ICD-10 CODE G44.51 TESTING

Magnetic resonance imaging (MRI) of the brain provides
the best information regarding the cranial vault and its con-
CLINICAL SYNDROME tents. MRI is highly accurate and helps identify abnormali-
Hemicrania continua is a primary headache disorder that is ties that may put the patient at risk for neurological disasters
classified as a trigeminal autonomic cephalgia that shares char- secondary to intracranial and brainstem pathological con-
acteristics of both cluster headache and migraine headache ditions, including tumors and demyelinating disease (Fig.
(Table 4.1). Similar to cluster headache, hemicrania continua 4.2). Magnetic resonance angiography (MRA) also may be
is a severe, unilateral headache with associated signs of auto- useful in identifying aneurysms, which may be responsi-
nomic dysfunction, including lacrimation, scleral injection, ble for the patient’s neurological findings. In patients who
eyelid ptosis, and nasal stuffiness syndrome (Fig. 4.1). In con- cannot undergo MRI, such as a patient with a pacemaker,
trast to cluster headache, which occurs 10 times more com- computed tomography (CT) is a reasonable second choice.
monly in men, hemicrania continua occurs more commonly in Radionuclide bone scanning and plain radiography are indi-
women, a characteristic it shares with migraine. Like migraine cated if fracture or bony abnormality such as metastatic
headache, hemicrania continua is associated with nausea and disease is considered in the differential diagnosis. Positron
vomiting, as well as sonophobia and phonophobia. Unlike the emission tomography may help further delineate and char-
pain of cluster and migraine headache, the pain of hemicrania acterize tumors responsible for the patient’s pain and neuro-
continua is continuous with intermittent severe exacerbations logical symptoms.
of pain. This pain is unilateral and is side locked (i.e., it does Screening laboratory tests consisting of complete blood cell
not change sides like migraine headache occasionally does). count, erythrocyte sedimentation rate, and automated blood
Hemicrania continua is an indomethacin responsive headache,
with complete resolution of headache and associated symptoms
with therapeutic doses of indomethacin. The cause of hemicra- TABLE 4.1 Comparison of Cluster Headache
nia continua is unknown, but like other trigeminal autonomic and Hemicrania Continua
cephalgias, functional magnetic resonance scanning and pos-
itron emission tomography reveal activation in the posterior Comparison Cluster Hemicrania Migraine
Factors Headache Continua Headache
hypothalamus during exacerbation of headaches.
Gender predominance Male Female Female
Absolute response to Negative Positive Negative
SIGNS AND SYMPTOMS indomethacin
Chronobiological Positive Negative Negative
During attacks of hemicrania continua, patients exhibit the fol-
pattern
lowing physical findings suggestive of Horner syndrome on the Alcohol trigger Positive Negative Sometimes
ipsilateral side of the pain: Length of attacks Longer Continuous, with Longer than cluster
• Conjunctival and scleral injection exacerbations headache
• Lacrimation Autonomic symptoms, Present Present Negative
• Nasal congestion lacrimation, scleral
• Rhinorrhea injection, eyelid pto-
• Ptosis of the eyelid sis, nasal stuffiness
As in cluster headache, the patient may become agitated syndrome
during attacks, rather than seeking dark and quiet as does the Photophobia Negative Positive Positive
Sonophobia Negative Positive Positive
patient with migraine. In contrast to cluster headache, alcohol
Nausea and vomiting Negative Positive Positive
consumption does not seem to trigger attacks of hemicrania
Continuous pain with Negative Positive Negative
continua. Between attacks, the neurological examination of a exacerbations
patient with hemicrania continua should be normal.
11
12 SECTION 1 Headache and Facial Pain Syndromes
Fig. 4.1 Hemicrania continua is a unilateral side-locked headache with associated signs of autonomic dys-
function. In contrast to cluster headache, which occurs primarily in men, hemicrania continua occurs primarily
in women.
chemistry testing should be performed if the diagnosis of hemi-
crania continua is in question. Intraocular pressure should be
measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Hemicrania continua is a clinical diagnosis supported by a
combination of clinical history, abnormal physical examina-
tion during exacerbation of baseline headache, radiography,
and MRI. Pain syndromes that may be confused for hemicra-
nia continua include cluster headache, chronic paroxysmal
hemicrania, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, ice pick headache,
and other indomethacin responsive headaches. Trigeminal
neuralgia involving the first division of the trigeminal nerve
is uncommon and is characterized by trigger areas and tic-
like movements. Demyelinating disease is generally associated
with other neurological findings, including optic neuritis and
other motor and sensory abnormalities. The pain of cluster
headache is episodic, whereas the pain of hemicrania conti-
nua is continuous with acute severe exacerbations (Fig. 4.3).
Cluster headache also has a male predominance, a chronobio-
logical pattern of attacks, and a lack of response to treatment
with indomethacin.
TREATMENT
Hemicrania continua uniformly responds to treatment with
indomethacin. Failure to respond to indomethacin puts the
diagnosis of hemicrania continua in question. A starting dose of
25 mg daily for 2 days and titrating to 25 mg three times per day
is a reasonable treatment approach. This dose may be carefully
increased up to 150 mg per day. Indomethacin must be used
carefully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to cyclo-
oxygenase-2 (COX-2) inhibitors in the treatment of hemicrania
continua have been noted in the headache literature. Underlying
sleep disturbance and depression are best treated with a tricyclic
antidepressant compound, such as nortriptyline, which can be
started at a single bedtime dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose hemicrania continua correctly may put the
patient at risk if intracranial pathological conditions or demy-
elinating disease, which may mimic the clinical presentation
of hemicrania continua, is overlooked. MRI is indicated in all
patients thought to have hemicrania continua. Failure to diag-
nose glaucoma, which may cause intermittent ocular pain, may
result in permanent loss of sight.
CLINICAL PEARLS Hemicrania continua is classified as a trigeminal
autonomic cephalgia. The trigeminal autonomic cephalgias are a group of
distinct headache syndromes that share a number of common and often over-
lapping clinical characteristics (Table 4.2). The diagnosis of hemicrania con-
tinua is made by obtaining a thorough, targeted headache history. Between
attacks, patients with hemicrania continua should have a normal neurological
examination. If the neurological examination is abnormal between attacks,
the diagnosis of hemicrania continua should be discarded and a careful search
for the cause of the patient’s neurological findings should be undertaken.
 CHAPTER 4 Hemicrania Continua 13

A B

C D
Fig. 4.2 A 57-Year-Old Woman With Recurrent GBM Postcontrast T1-weighted image (A) and rCBV map
(B) demonstrate two adjacent necrotic, peripherally enhancing lesions with elevated rCBV (white arrow) con-
sistent with recurrent GBM. After subtotal reresection of the peripheral lesion and 2 weeks of treatment
with TMZ and bevacizumab, postcontrast T1-weighted image (C) and rCBV map (D) show reduced size and
decreased intensity of enhancement of the residual medial necrotic rim-enhancing lesion with no significant
rCBV elevation (white arrow). Findings are consistent with response to treatment. The patient had a rela-
tively long OS (>17 months) after bevacizumab initiation. (From Boxerman JL, Shiroishi MS, Ellingson BM,
et al. Dynamic susceptibility contrast MR imaging in glioma: review of current clinical practice. Magn Reson
Imaging Clin N Am. 2016;24(4):649–670.) rCBV, Cerebral blood volume; GBM, glioblastoma multiforme; OS,
overall survival rate; TMZ, temozolomide.

SUNCT Paroxysmal Hemicrania Daily persistent

hemicrania continua headache

5 s–4 min 2–30 min 15–180 min Time

Continuous baseline headache Continuous

with acute exacerbations
Fig. 4.3 The Temporal Relationships of Trigeminal Autonomic Cephalalgias. (Modified from Silberstein
SD, Vodovskaia N. Trigeminal autonomic cephalalgias other than cluster headache. Med Clin North Am.
2013;97(2):321–328.)
14 SECTION 1 Headache and Facial Pain Syndromes

TABLE 4.2 Characteristics of the Trigeminal SUGGESTED READINGS

Autonomic Cephalgias Benitez-Rosario MA, McDarby G, Doyle R, Fabby C. Chronic clus-
• Unilateral ter-like headache secondary to prolactinoma: uncommon cephal­
• Short duration algia in association with brain tumors. J Pain Symptom Manage.
• High frequency 2009;47:271–276.
• Orbital, periorbital, or temporal Evans RW. Case studies of uncommon and rare headache disorders.
• Associated autonomic symptoms Neurol Clin. 2016;34(3):631–650.
• Lacrimation Jay GW, Barkin RL. Primary headache disorders, Part I: migraine and
• Conjunctival injection the trigeminal autonomic cephalalgias. Dis Mon. 2017;63(11):308–
• Nasal congestion 338.
• Ptosis Schytz HW, Hargreaves R, Ashina M. Challenges in developing drugs
• Eyelid edema for primary headaches. Prog Neurobiol. 2017;152:70–88.
Silberstein SD, Vodovskaia N. Trigeminal autonomic cephalalgias oth-
er than cluster headache. Med Clin North Am. 2014;97(2):421–428.

ICD-10 CODE G50.0
CLINICAL SYNDROME
Charlin syndrome, also known as nasociliary neuralgia and
Charlin-Sluder cephalgia, is an uncommon cause of head and
face pain. As with most headache syndromes, the exact cause
of the pain of Charlin syndrome is unknown. However, the
pathogenesis of this uncommon cause of head and face pain is
thought to be dysfunction of the nasociliary ganglion in a man-
ner analogous to the dysfunction of the sphenopalatine gan-
glion thought to be the source of cluster headache. The pain of
Charlin syndrome has a rapid onset to peak, with attacks lasting
45 to 60 minutes. In some patients, these attacks can be trig-
gered by sensory stimulation of the affected areas. Although in
many ways similar to cluster headache (e.g., retroorbital loca-
tion of pain, profuse unilateral rhinorrhea, rapid onset to peak,
and short duration of attacks), many dissimilarities also exist.
In contrast to cluster headache, alcohol consumption does not
appear to trigger attacks of Charlin syndrome and the seasonal
and chronobiological patterns so characteristic of cluster head-
ache do not seem to be a factor (Table 5.1). Blockade of the
sphenopalatine ganglion, which is so effective in the treatment
of cluster headache, is of little value in the treatment of Charlin
syndrome. Patients suffering from Charlin syndrome uniformly
respond to daily nasociliary nerve blocks with local anesthetic,
as described subsequently.
SIGNS AND SYMPTOMS
Patients suffering from Charlin syndrome present with the
complaint of severe paroxysms of ocular or retroorbital pain
that radiates into the ipsilateral forehead, nose, and maxillary
region. This pain is associated with voluminous ipsilateral rhi-
norrhea and congestion of the nasal mucosa and significant
inflammation of the affected eye (Fig. 5.1).
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters secondary
5
Charlin Syndrome
to intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease (Fig. 5.2). Magnetic
resonance angiography (MRA) also may be useful in helping
identify aneurysms, which may be responsible for the patient’s
neurological findings. In patients who cannot undergo MRI,
such as a patient with a pacemaker, computed tomography (CT)
is a reasonable second choice. Radionuclide bone scanning and
plain radiography are indicated if fracture or bony abnormal-
ity such as metastatic disease is considered in the differential
diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
Charlin syndrome is in question. Intraocular pressure should
be measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Charlin syndrome is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic Charlin
syndrome include cluster headache, temporal arteritis, trigemi-
nal neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania
(see Table 5.1). Trigeminal neuralgia involving the first division
of the trigeminal nerve is uncommon and is characterized by
trigger areas and tic-like movements. Demyelinating disease is
generally associated with other neurological findings, including
optic neuritis and other motor and sensory abnormalities. The
pain of chronic paroxysmal hemicrania lasts much longer than
the pain of Charlin syndrome.
TREATMENT
The treatment of Charlin syndrome is analogous to the treat-
ment of trigeminal neuralgia. The use of anticonvulsants such as
carbamazepine and gabapentin represents a reasonable starting
point. High-dose steroids tapered over 10 days also have been
anecdotally reported to provide relief. For patients who do not
respond to the previously mentioned treatments, daily nasocil-
iary ganglion block with local anesthetic and steroid is a rea-
sonable next step. Underlying sleep disturbance and depression
associated with the pain of supraorbital neuralgia are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
15
16 SECTION 1 Headache and Facial Pain Syndromes

TABLE 5.1 Comparison of Cluster Headache

and Charlin Syndrome
Cluster Charlin
Comparison Factors Headache Syndrome
Ocular and retroorbital location Yes Yes
Unilateral Yes Yes
Rapid onset to peak Yes Yes
Severe intensity Yes Yes
Attacks occur in paroxysms Yes Yes
Duration of attacks short Yes Yes
Pain free between attacks Yes Yes
Significant rhinorrhea during attacks Yes Yes
Alcohol triggers attacks Yes No
Tactile trigger areas No Yes
Seasonal pattern of attacks Yes No
Chronobiological pattern of attacks Yes No
Significant eye inflammation No Yes
Responds to sphenopalatine ganglion Yes No Fig. 5.2 Multiple Sclerosis Fluid-attenuated inversion recovery (FLAIR)
block parasagittal MR image depicts the extensive demyelinated plaques of
Responds to nasociliary block No Yes progressive multiple sclerosis. (From Haaga JR, Lanzieri CF, Gilkeson
RC, eds. CT and MR Imaging of the Whole Body. 4th ed. Philadelphia:
Mosby; 2003:466.)

COMPLICATIONS AND PITFALLS

Failure to diagnose Charlin syndrome correctly may put the
patient at risk if an intracranial pathological condition or demy-
elinating disease, which may mimic the clinical presentation
of Charlin syndrome, is overlooked. MRI is indicated in all
patients thought to have Charlin syndrome. Failure to diagnose
glaucoma or temporal arteritis, which also may cause intermit-
tent ocular pain, may result in permanent loss of sight.

CLINICAL PEARLS Nasociliary nerve block via the medial orbital

approach is especially useful in the diagnosis and palliation of pain secondary
to Charlin syndrome. Given the uncommon nature of this headache syndrome
and its overlap with the symptoms of cluster headache and other neurological
problems, including cavernous sinus thrombosis and intracranial and retro-
orbital tumors, Charlin syndrome must remain a diagnosis of exclusion. All
patients suspected to have Charlin syndrome require MRI of the brain with and
without gadolinium contrast material and thorough ophthalmological and neu-
rological evaluation. Nasociliary nerve block via the medial orbital approach
should be performed only by clinicians familiar with the regional anatomy.

Fig. 5.1 Patients suffering from Charlin syndrome present with the
complaint of severe paroxysms of ocular or retroorbital pain that radi-
ates into the ipsilateral forehead, nose, and maxillary region. The pain is
associated with voluminous ipsilateral rhinorrhea and congestion of the
nasal mucosa and significant inflammation of the affected eye.
SUGGESTED READINGS
Becker M, Kohler R, Vargas MI, Viallon M, Delavelle J. Pathology of
the trigeminal nerve. Neuroimaging Clin N Am. 2008;18:283–307.
Craven J. Anatomy of the cranial nerves. Anaesth Intensive Care Med.
2010;11:529–534.
Lewis DW, Gozzo YF, Avner MT. The “other” primary headaches in
children and adolescents [review]. Pediatr Neurol. 2005;33:303–313.
Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
view. Philadelphia: Saunders; 2009:15–17.
Waldman SD. Charlin’s syndrome. In: Waldman SD, ed. Atlas of
Pain Management Injection Techniques. Philadelphia: Saunders;
2007:20–24.
 6
Sexual Headache

ICD-10 CODE R51
THE CLINICAL SYNDROME
Sexual headache, which is also known as primary headache
associated with sexual activity, is a term used to describe a
group of headaches associated with sexual activity. Clinicians
have identified the following three general types of headache
associated with sexual activity:
• Explosive type
• Dull type
• Postural type
Each of these sexual headache types was previously called
benign coital headache, but this term has been replaced by sex-
ual headache because each may occur with sexual activity other
than coitus (Fig. 6.1). In general, sexual headache includes a
benign group of disorders, but a rare patient may have acute
subarachnoid hemorrhage during sexual activity, which may be
erroneously diagnosed as the benign explosive type of sexual
headache. There is no gender predilection for sexual headache,
and the occurrence of all types of sexual headache may be epi-
sodic rather than chronic. Rarely, more than one type of sexual
headache occurs in the same patient. Sexual headaches have
been associated with the use of cannabis, pseudoephedrine, oral
contraceptives, and amiodarone.
occipital, but some patients volunteer that the pain felt “like the
top of my head was going to blow off.” The pain is usually bilat-
eral, but isolated cases of unilateral explosive sexual headache
have been reported. The pain usually remains intense for 10 to
15 minutes and then gradually abates. Some patients note some
residual headache pain for 2 days.
Dull Type of Sexual Headache
The dull type of sexual headache begins during the early por-
tion of sexual activity. This headache type has an aching char-
acter and begins in the occipital region. The headache becomes
holocranial as sexual activity progresses toward orgasm. It may
peak at orgasm, but in contrast to the explosive type of sexual
headache, the dull type disappears rapidly after orgasm. Ceasing
sexual activity usually aborts the dull type of sexual headache.
Some headache specialists think the dull type of sexual head-
ache is simply a milder version of the explosive type of sexual
headache.
Postural Type of Sexual Headache
The postural type of sexual headache is similar to the explosive
type of sexual headache in that it occurs just before or during
orgasm. Its rapid onset to peak and severe intensity also are sim-
ilar to that of the explosive type. It differs from the explosive
type of headache in that the headache symptoms recur when the

SIGNS AND SYMPTOMS

Patients with sexual headache present differently depending on
the type of sexual headache experienced. Each clinical presen-
tation is discussed subsequently.

Explosive Type of Sexual Headache

The explosive type of sexual headache is the most common type
of sexual headache encountered in clinical practice. The patient
usually fears he or she has had a stroke. The patient may be less
forthcoming about the circumstances surrounding the onset of
headache, and tactful questioning may be required to ascertain
the actual clinical history. The explosive type of sexual headache
occurs suddenly, with an almost instantaneous onset to peak
just before or during orgasm. The intensity of the explosive type
of sexual headache is severe and has been likened to the pain of Fig. 6.1 Sexual headaches show no gender predilection and are gen-
acute subarachnoid hemorrhage. The location of pain is usually erally benign.

17
18 SECTION 1 Headache and Facial Pain Syndromes

Fig. 6.2 Brain magnetic resonance angiography performed 1 month

after sexual headache onset showing multifocal vasoconstriction in
the left posterior cerebral artery (arrows). (From Hu CM, Lin YJ, Fan
YK, et al. Isolated thunderclap headache during sex: orgasmic head-
ache or reversible cerebral vasoconstriction syndrome? J Clin Neu-
rosci. 2010;17[10]:1349–1351, fig. 1, ISSN 0967-5868, https://doi.
org/10.1016/j.jocn.2010.01.052. http://www.sciencedirect.com/science/ A
article/pii/S0967586810002419.)

patient stands up, in a manner analogous to postdural puncture

headache. The postural component of this type of sexual head-
ache is thought to be due to minute tears in the dura that may
occur during intense sexual activity.

TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters secondary
to intracranial and brainstem pathological conditions, including
tumors, demyelinating disease, and hemorrhage. More impor-
tantly, MRI helps identify bleeding associated with leaking intra-
cranial aneurysms. Magnetic resonance angiography (MRA)
and cerebral arteriography may be useful in helping identify
aneurysms or other arterial abnormalities responsible for the
patient’s neurological symptoms (Figs 6.2 and 6.3). In patients B
who cannot undergo MRI, such as patients with pacemakers,
Fig. 6.3 Cerebral angiogram revealed segmental (boundaries denoted
computed tomography (CT) is a reasonable second choice. Even
by large arrows) irregularities (small arrows) of the basilar artery in
if blood is not present on MRI or CT, if intracranial hemorrhage both anterior-posterior (A) and lateral (B) views. (From Delasobera BE,
is suspected, lumbar puncture should be performed. Osborn SR, Davis JE. Thunderclap headache with orgasm: a case of
Screening laboratory tests consisting of complete blood cell basilar artery dissection associated with sexual intercourse. J Emerg
count, erythrocyte sedimentation rate, and automated blood Med. 2012;43[1]:e43–e47, fig. 1.)
chemistry testing should be performed if the diagnosis of sexual
headache is in question. Intraocular pressure should be mea- cluster headache, migraine, and chronic paroxysmal hemi-
sured if glaucoma is suspected. crania. Trigeminal neuralgia involving the first division of the
trigeminal nerve is uncommon and is characterized by trigger
areas and tic-like movements. Demyelinating disease is gener-
DIFFERENTIAL DIAGNOSIS ally associated with other neurological findings, including optic
Sexual headache is a clinical diagnosis supported by a combi- neuritis and other motor and sensory abnormalities. The pain of
nation of clinical history, normal physical examination, radi- chronic paroxysmal hemicrania and cluster headache is associ-
ography, MRI, and MRA. Pain syndromes that may mimic ated with redness and watering of the ipsilateral eye, nasal con-
sexual headache include trigeminal neuralgia involving the gestion, and rhinorrhea during the headache. These findings are
first division of the trigeminal nerve, demyelinating disease, absent in all types of sexual headache. Migraine headache may

or may not be associated with nonpainful neurological findings
known as aura, but the patient almost always reports some sys-
temic symptoms, such as nausea or photophobia, not typically
associated with sexual headache.
TREATMENT
It is generally thought that avoiding the inciting activity for a
few weeks decreases the propensity to trigger sexual headaches.
If this avoidance technique fails or is impractical because of
patient preference, a trial of propranolol is a reasonable next
step. A low dose of 20 to 40 mg as a daily dose and titrating
in 20-mg increments to 200 mg as a divided daily dose until
prophylaxis occurs treats most patients suffering from sexual
headache. Propranolol should be used with caution in patients
with asthma or cardiac failure and patients with brittle diabetes.
If propranolol is ineffective, indomethacin may be tried. A
starting dose of 25 mg daily for 2 days and titrating to 25 mg
three times per day is a reasonable treatment approach. This dose
may be carefully increased to 150 mg per day. Indomethacin
must be used carefully, if at all, in patients with peptic ulcer dis-
ease or impaired renal function. Anecdotal reports of a positive
response to cyclooxygenase-2 (COX-2) inhibitors and magne-
sium in the treatment of sexual headache have been noted in the
headache literature. Underlying sleep disturbance and depres-
sion are best treated with a tricyclic antidepressant compound,
such as nortriptyline, which can be started at a single bedtime
dose of 25 mg.
COMPLICATIONS AND PITFALLS
Failure to diagnose sexual headache correctly may put the
patient at risk if intracranial pathology or demyelinating
disease, which may mimic the clinical presentation of sexual
CHAPTER 6 Sexual Headache 19
headache, is overlooked. MRI, MRA, and occasionally cerebral
angiography are indicated in all patients thought to have sex-
ual headache. Failure to diagnose glaucoma, which also may
cause intermittent ocular pain, may result in permanent loss
of sight.
CLINICAL PEARLS The diagnosis of sexual headache is made by obtain-
ing a thorough, targeted headache history. As mentioned earlier, patients may
not be forthcoming about the events surrounding the onset of their headache,
and the clinician should be sensitive to this fact. Patients suffering from sexual
headache should have a normal neurological examination. If the neurological
examination is abnormal, the diagnosis of sexual headache should be dis-
carded and a careful search for the cause of the patient’s neurological findings
should be undertaken.
SUGGESTED READINGS
Delasobera BE, Osborn SR, Davis JE. Thunderclap headache with
orgasm: a case of basilar artery dissection associated with sexual
intercourse. J Emerg Med. 2012;43(1):e43–e47.
Evans RW. Diagnostic testing for migraine and other primary head-
aches. Neurol Clin. 2009;27:393–415.
Hu CM, Lin YJ, Fan YK, et al. Isolated thunderclap headache during
sex: orgasmic headache or reversible cerebral vasoconstriction
syndrome? J Clin Neurosci. 2010;17:1349–1351.
Jolobe OMP. The differential diagnosis includes reversible cerebral
vasoconstrictor syndrome. Am J Emerg Med. 2010;28:637.
Kim HJ, Seo SY. Recurrent emotion-triggered headache following
primary headache associated with sexual activity. J Neurol Sci.
2008;273:142–143.
Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exer-
tional and sexual headaches in a Turkish population of headache
patients. J Clin Neurosci. 2008;15:774–777.
 7
Cough Headache
ICD-10 CODE R51
THE CLINICAL SYNDROME
Cough headache is a term used to describe headaches triggered
by coughing and other activities associated with a Valsalva
maneuver, such as laughing, straining at stool, lifting, and bend-
ing the head toward the ground (Fig. 7.1). Clinicians have iden-
tified the following two types of cough headache:
• Benign primary cough headache
• Symptomatic cough headache
Initially, both types of cough headache were thought to be
related to sexual and exertional headaches, but they are now
considered distinct clinical entities. A strong male predilection
is seen for benign cough headache and no gender predilection
for symptomatic cough headache.
SIGNS AND SYMPTOMS
Patients suffering from cough headache present differently
depending on the type of cough headache experienced. Each
clinical presentation is discussed.
Benign Cough Headache
Benign cough headache is not associated with obvious neuro-
logical or musculoskeletal disease. More than 80% of patients
with benign cough headache are males, in contradistinction to
symptomatic cough headache, in which no gender predilection
is seen. The onset of benign cough headache is abrupt, occur-
ring immediately after coughing or other activities that cause
a Valsalva maneuver. Although the intensity of pain is severe
and peaks rapidly, it lasts only seconds to minutes. The charac-
ter of the pain associated with benign cough headache is split-
ting or sharp, and the pain is in the occipital region bilaterally
and occasionally the vertex of the skull. No accompanying neu-
rological or systemic symptoms are seen, as with cluster and
migraine headaches. The age of onset of benign cough head-
ache is generally in the late fifth or sixth decade of life. If such
headaches occur before age 50, there should be strong clinical
suspicion that the patient either has symptomatic cough head-
ache or a pathological condition in the posterior fossa, such as
Arnold-Chiari malformation or tumor. Tumors of the foramen
20
magnum also may mimic the presentation of benign cough
headache even if no neurological symptoms are present.
Symptomatic Cough Headache
Symptomatic cough headache is almost always associated with
structural abnormalities of the cranium, such as Arnold-Chiari
malformation I and II or intracranial tumors (Fig. 7.2). The
symptoms associated with symptomatic cough headache are
thought to be due to herniation of the cerebellar tonsil through
the foramen magnum into the space normally occupied by the
upper portion of the cervical spinal cord. Similar to benign
cough headache, the onset of pain associated with symptomatic
cough headache is abrupt, occurring immediately after cough-
ing or other activities that cause a Valsalva maneuver. Although
the intensity of pain is severe and peaks rapidly, it lasts only
seconds to minutes. In contrast to benign cough headache,
associated neurological symptoms may be present, including
difficulty swallowing, faintness, and numbness in the face and
upper extremities. These associated symptoms should be taken
very seriously because they are indicative of increased intracra-
nial pressure and herniation of the intracranial contents.
The character of the pain associated with symptomatic cough
headache is splitting or sharp, and pain is in the occipital region
bilaterally and occasionally the vertex of the skull. The age of
onset of symptomatic cough headache is generally in the third
decade of life, although, depending on the amount of neurolog-
ical compromise, it may occur at any age. In contrast to benign
cough headache, which occurs predominantly in men, symp-
tomatic cough headache occurs with equal prevalence in both
genders.
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters second-
ary to intracranial and brainstem pathological conditions,
including tumors and demyelinating disease. Special attention
to the foramen magnum may help identify more subtle abnor-
malities responsible for posterior fossa neurological signs and
symptoms. MRI helps identify bleeding associated with leaking
intracranial aneurysms, which may mimic the symptoms of
both types of cough headache. Magnetic resonance angiography

Herniation of
cerebellar tonsil
Spinal cord
Fig. 7.1 Symptomatic cough headache is often associated with structural abnormalities, such as Arnold-
Chiari malformation, and usually occurs in the third decade of life.
Fig. 7.2 Low-lying cerebellar tonsils (straight arrows) of a Chiari mal-
formation are shown deforming the medulla (curved arrow) in a sagit-
tal T1-weighted spin echo image. 4, Fourth ventricle. (From Stark DD,
Bradley WG Jr, eds. Magnetic Resonance Imaging. 3rd ed. St Louis:
Mosby; 1999:1841.)
CHAPTER 7 Cough Headache 21
(MRA) may be useful in helping identify aneurysms responsi-
ble for the patient’s neurological symptoms. In patients who
cannot undergo MRI, such as patients with pacemakers, com-
puted tomography (CT) is a reasonable second choice. Lumbar
puncture should be performed if intracranial hemorrhage is
suspected, even if blood is not present on MRI or CT. Plain
radiographs of the cervical spine also may be useful in the eval-
uation of Arnold-Chiari malformations and should be included
in the evaluation of all patients with cough headache.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of cough
headache is in question. Intraocular pressure should be mea-
sured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
Cough headache is a clinical diagnosis supported by a combina-
tion of clinical history, physical examination, radiography, MRI,
and MRA. Pain syndromes that may mimic cough headache
include benign exertional headache, ice pick headache, sexual
headache, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, cluster headache,
and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
ments. Demyelinating disease is generally associated with other
neurological findings, including optic neuritis and other motor
and sensory abnormalities. The pain of chronic paroxysmal
hemicrania and cluster headache is associated with redness and
watering of the ipsilateral eye, nasal congestion, and rhinorrhea
during the headache. These findings are absent in all types of
22 SECTION 1 Headache and Facial Pain Syndromes

cough headache. Migraine headache may or may not be associ-
ated with painless neurological findings known as aura, but the
patient almost always reports some systemic symptoms, such
as nausea or photophobia, not typically associated with cough
headache.
TREATMENT
Indomethacin is the treatment of choice for benign cough head-
ache. A starting dose of 25 mg daily for 2 days and titrating to
25 mg three times per day is a reasonable treatment approach.
This dose may be carefully increased up to 150 mg per day.
Indomethacin must be used carefully, if at all, in patients with
peptic ulcer disease or impaired renal function. Headache spe-
cialists have noted anecdotal reports of a positive response to
cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign
cough headache. Underlying sleep disturbance and depression
are best treated with a tricyclic antidepressant compound, such
as nortriptyline, which can be started at a single bedtime dose
of 25 mg.
The only uniformly effective treatment for symptomatic
cough headache is surgical decompression of the foramen mag-
num. This surgery is usually done via suboccipital craniectomy.
Surgical decompression prevents the low-lying cerebellar ton-
sils from obstructing the flow of spinal fluid from the cranium
to the spinal subarachnoid space during a Valsalva maneuver.
COMPLICATIONS AND PITFALLS
Failure to diagnose cough headache correctly may put the
patient at risk if intracranial pathology or demyelinating dis-
ease, which may mimic the clinical presentation of cough head-
ache, is overlooked. MRI and MRA are indicated in all patients
thought to have cough headache. Failure to diagnose glaucoma,
which also may cause intermittent ocular pain, may result in
permanent loss of sight.
SUGGESTED READINGS
Berciano J, Poca M-A, García A, Sahuquillo J. Paroxysmal cervi-
cobrachial cough-induced pain in a patient with syringomy-
elia extending into spinal cord posterior gray horns. J Neurol.
2007;54:678–681.
Chen YY, Lirng JF, Fuh JL, et al. Primary cough headache is associat-
ed with posterior fossa crowdedness: a morphometric MRI study.
Cephalalgia. 2004;24:694–699.
Rothrock JF. Headaches caused by vascular disorders. Neurol Clin.
2014;32(2):305–319.
Cutrer M, DeLange FJ. Cough, exercise, and sex headaches. Neurol
Clin. 2014;32(2):433–450.
Langridge B, Phillips E, Choi D. Chiari malformation type 1: a sys-
tematic review of natural history and conservative management.
World Neurosurg. 104:213–219
Pascual J, Rubén Martín A, Oterino A. Headaches precipitated by
cough, prolonged exercise or sexual activity: a prospective etiolog-
ical and clinical study. J Headache Pain. 2008;9:259–266.
Pascual J. Primary cough headache. Curr Pain Headache Rep.
2005;9:272–276.
Pascual J. Cough and exertional headache, primary. In: Encyclopedia
of the Neurological Sciences. 2nd ed. Oxford: Academic Press;
2014:881–884.
Waldman SD. Arnold Chiari malformation type I. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:27–28.
Waldman SD. Arnold Chiari malformation type II. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:29–30.

CLINICAL PEARLS Any patient presenting with headaches associated

with exertion or Valsalva maneuver should be taken very seriously. Although
statistically most of these headaches ultimately are proved to be of benign
cause, a few patients have potentially life-threatening disease. The diagno-
sis of cough headache is made by obtaining a thorough, targeted headache
history and performing a careful physical examination. The clinician must sep-
arate patients suffering from benign cough headache from patients suffering
from symptomatic cough headache. Patients with benign cough headache
should have a normal neurological examination. If the neurological examina-
tion is abnormal, the diagnosis of benign cough headache should be discarded
and a careful search for the cause of the patient’s neurological findings should
be undertaken.

Sudden Unilateral Neuralgiform
Conjunctival Injection
ICD-10 CODE G50.0
THE CLINICAL SYNDROME
Sudden unilateral neuralgiform conjunctival injection tearing
(SUNCT) headache is an uncommon primary headache disor-
der that is one of a group of three headache syndromes known
as the trigeminal autonomic cephalgias (Table 8.1). Whether
SUNCT headache is in fact a distinct headache entity or simply
a constellation of symptoms that occurs on a continuum along
with the other trigeminal autonomic cephalgias is a point of
ongoing debate among headache and pain management spe-
cialists (Fig. 8.1). As with most headache syndromes, the exact
cause of the pain of SUNCT headache is unknown; however, the
pathogenesis of this uncommon cause of head and face pain is
thought to be dysfunction of the trigeminal autonomic reflex.
The pain of SUNCT headache has a rapid onset to peak,
with attacks lasting 5 seconds to 4 minutes and the frequency
of attacks ranging from 20 to 200 attacks per day. In some
patients, these attacks can be triggered by sensory stimulation
of the affected areas, such as when washing the face, brushing
the teeth, and so forth. Although in many ways similar to clus-
ter headache (e.g., unilateral, periorbital, and frontal location
of pain, sclera injection, rapid onset to peak, short duration of
attacks, and pain-free periods between attacks), SUNCT exhib-
its many dissimilarities as well. In contrast to cluster head-
ache, alcohol consumption does not seem to trigger attacks of
SUNCT headache, and there do not seem to be the seasonal
and chronobiological patterns so characteristic of cluster head-
ache, although SUNCT headache occurs most frequently in the
morning and afternoon (Table 8.2).
Blockade of the sphenopalatine ganglion, which is so effec-
tive in the treatment of cluster headache, is of little value in the
treatment of SUNCT headache. Patients suffering from SUNCT
headache may respond to daily trigeminal nerve blocks with
local anesthetic, as described subsequently.
SIGNS AND SYMPTOMS
Patients with SUNCT headache present with the complaint
of severe paroxysms of ocular or periorbital pain that radi-
ate into the ipsilateral temple, forehead, nose, cheek, throat,
8
Tearing Headache
and maxillary region. This pain is associated with significant
inflammation of the affected eye and associated autonomic
signs and symptoms (Figs 8.2 and 8.3). The pain is neural-
giform and severe to excruciating in intensity (Table 8.3).
SUNCT occurs on the right side 70% of the time in a manner
analogous to trigeminal neuralgia. Like trigeminal neuralgia,
rare cases of bilateral SUNCT headache have been reported.
Also, like trigeminal neuralgia, the pain of SUNCT headache
rarely switches sides. SUNCT headache occurs slightly more
frequently in males. It can occur at any age, with a peak inci-
dence in the fifth decade.
TESTING
Magnetic resonance imaging (MRI) of the brain provides
the clinician with the best information regarding the cra-
nial vault, vasculature, and its contents (Fig. 8.4). MRI is
highly accurate and helps identify abnormalities that may
put the patient at risk for neurological disasters secondary to
intracranial and brainstem pathological conditions, includ-
ing tumors and demyelinating disease. Magnetic resonance
angiography (MRA) also may be useful in helping iden-
tify aneurysms, which may be responsible for the patient’s
TABLE 8.1 Trigeminal Autonomic
Cephalgias
Cluster headache
Chronic paroxysm hemicranias
Sudden unilateral neuralgiform conjunctival injection tearing headache
Paroxysmal Cluster
SUNCT hemicrania headache
5 s–4 min 2–30 min 15–180 min Time
Overlap Overlap
between duration between duration
Fig. 8.1 Overlap Between Attack Duration in Trigeminal Autonomic
Cephalalgias SUNCT, Sudden unilateral neuralgiform conjunctival injec-
tion tearing. (From Leone M, Bussone G. Pathophysiology of trigeminal
autonomic cephalalgias. Lancet Neurol. 2009;8:755–774.)
23
24 SECTION 1 Headache and Facial Pain Syndromes

TABLE 8.2 Comparison of Cluster Headache

and Sudden Unilateral Neuralgiform FF
Conjunctival Injection Tearing Headache
Cluster SUNCT
Comparison Factors Headache Headache
Ocular and retroorbital location Yes Yes CI, Mi
Unilateral Yes Yes
Rapid onset to peak Yes Yes
Severe intensity Yes Yes
Attacks occur in paroxysms Yes Yes
Duration of attacks short Yes Yes EO, Pt
Pain free between attacks Yes Yes
Significant rhinorrhea during attacks Yes No
Alcohol triggers attacks Yes No
Tactile trigger areas No Yes
Seasonal pattern of attacks Yes No
NC, Rh
Chronobiological pattern of attacks Yes No La
Significant eye inflammation No Yes
Responds to sphenopalatine ganglion block Yes No
Responds to trigeminal nerve block No Yes

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.
Fig. 8.3 Autonomic signs and symptoms, ipsilateral to the pain,
observed in a patient with sudden unilateral neuralgiform conjunctival
injection tearing. CI, Conjunctival injection; EO, eyelid edema; FF, fore-
head and facial flushing; La, lacrimation; Mi, miosis; NC, nasal conges-
tion; Pt, ptosis; Rh, rhinorrhea. (From Kitahara I, Fukuda A, Imamura Y,
et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome.
World Neurosurg. 2015;84[4]:1080–1083, fig. 1.)

TABLE 8.3 Descriptors of Pain Associated

With Sudden Unilateral Neuralgiform
Conjunctival Injection Tearing Headache
Stabbing
Shooting
Lancinating
Shock-like
Sharp
Piercing
Pricking
Staccato-like

Fig. 8.2 Patients with sudden unilateral neuralgiform conjunctival

injection tearing headache present with severe paroxysms of ocular or
periorbital pain that radiates into the ipsilateral temple, forehead, nose,
cheek, throat, and maxillary region that is associated with significant
inflammation of the affected eye.
neurological findings. In patients who cannot undergo MRI,
such as patients with pacemakers, computed tomography
(CT) is a reasonable second choice. Radionuclide bone scan-
ning and plain radiography are indicated if fracture or bony
abnormality such as metastatic disease is considered in the
differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of
SUNCT headache is in question. Intraocular pressure should be
measured if glaucoma is suspected.
DIFFERENTIAL DIAGNOSIS
SUNCT headache is a clinical diagnosis supported by a com-
bination of clinical history, normal physical examination, radi-
ography, and MRI. Pain syndromes that may mimic SUNCT
headache include cluster headache, temporal arteritis, trigem-
inal neuralgia involving the first division of the trigeminal
nerve, demyelinating disease, primary stabbing headache, hyp-
nic headache, and chronic paroxysmal hemicrania, although
because of the overlapping features of all headache and facial
pain syndromes, SUNCT headache can be easily mistaken for
another type of headache or facial pain (Fig. 8.5; Table 8.4).
Trigeminal neuralgia involving the first division of the trigem-
inal nerve is uncommon and is characterized by trigger areas
and tic-like movements. Demyelinating disease is generally
associated with other neurological findings, including optic
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POSITIVES, COMPARATIVES, SUPERLATIVES.

239.
Pos., A pronoun; Com., A period of time; Sup., Fermenting froth.
Answer

240.
Pos., A knot of ribbon; Com., An animal; Sup., Self-praise.
Answer

241.
Pos., A reward; Com., Dread; Sup., A festival.
Answer

242.
Pos., To reward; Com., A fruit; Sup., An adhesive mixture.
Answer

243.
Pos., A meadow; Com., An unfortunate king; Sup., The smallest.
Answer
 244.

Pos., In a regular line;

Com., With an appetite fine;
Sup., ’Twill be done when we dine.

Answer

245.

Pos., Busy, noisy, and cheerful.

Com., The thought of it saddening and tearful;
Sup., Its roar and its fierce claws are fearful.

Answer

246.

Pos., The end of all time;

Com., Judge of music and rhyme;
Sup., The Orient clime.

Answer

247.

Pos., Denotes a bond or tie;

Com., In the centre it doth lie;
Sup., The billows break on it and die.
 Answer

248.
Pos., An American genius; Com., To turn out or to flow; Sup., An
office, an express, a place, a piece of timber.
Answer

249.
Pos., To depart; Com., To wound; Sup., A visible spirit.
Answer

250.

Pos., Pleasant, dreary, wet or dry;

Com., If ’tis light or heavy, try,
On your scales, before you buy;
Sup., Don’t spend money foolishly!

Answer

251.
A gentleman who had sent to a certain city for a car-load of fuel,
wrote thus to his nephew residing there:
“Dear Nephew
;
 Uncle John.”
Presently he received the following reply:
“Dear Uncle
:
James.”
Answer

252.
Why is a man up stairs, stealing, like a perfectly honorable man?
Answer

253.
Why is a ship twice as profitable as a hen?
Answer

254.
Why can you preserve fruit better by canning it, than in any other
way?
Answer

255.

Twelve kinds of things in fact, not fiction,

Behind a veil of contradiction.
 * * * * *
All dressed in silk, with stately grace,
We stand with ready ears,
And yet the sounds that greet the place
Not one among us hears.1
We’re keen and quick our holes to find
And run in lively mood,
And yet we’re footless quite and blind,
Although our eyes are good.2
Our perfect heads can’t give us sense,
Though we are naught without them;3
Our useful tongues are mere pretense—
No talk or taste about them.4
Our locks though fine can ne’er be combed;5
Our teeth can never bite;6
Our mouths from out our heads have roamed,
And oft outgrow them quite.7
Our hearts no pity have, or joy,
Yet they’re our richest worth;8
Our hands ne’er waved at girl or boy,
Or anything on earth.9
Alive are we, yet buried quite;
Our trust is in our eyes;
They help us out through darkest night,
Though sight stern fate denies.10
We sally forth when day is done,
And set the owls a-hooting,
And, though we have no bow or gun,
We often go a-shooting.11
Our souls, alas! are dull and low,
Down-trodden, from the start;
Yet who shall say, in weal or wo,
They’re not our better part?12

Answer

256.
 Within this world a creature once did dwell,
As sacred writings unto us do tell,
Who never shall be doomed to Satan’s home,
Nor unto God’s celestial Kingdom come;
Yet in him was a soul that either must
Suffer in Hell, or reign among the just.

Answer

257.
What best describes, and most impedes, a pilgrim’s progress?
Answer

258.
Why is a girl not a noun?
Answer

259.
What part of their infant tuition have old maids and old bachelors
most profited by?
Answer

260.
What is that which never asks any questions, and yet requires
many answers?
 Answer

261.
What quadrupeds are admitted to balls, operas, and dinner-
parties?
Answer

262.
If a bear were to go into a linen-draper’s shop, what would he
want?
Answer

263.
When does truth cease to be truth?
Answer

264.
How many dog-stars are there?
Answer

265.
What is worse than raining cats and dogs?
 Answer

266.
Why is O the only vowel that can be heard?
Answer

267.
Why is a man that has no children invisible?
Answer

268.
What is it which has a mouth, and never speaks; a bed, and
never sleeps?
Answer

269.
Which burns longer, a wax or sperm candle?
Answer

270.
Why is a watch like an extremely modest person?
Answer

271.

LORD MACAULAY’S LAST RIDDLE.

Let us look at it quite closely,

’Tis a very ugly word,
And one that makes me shudder
Whenever it is heard.
It mayn’t be very wicked;
It must be always bad,
And speaks of sin and suffering
Enough to make one mad.
They say it is a compound word,
And that is very true;
And, when they decompose it,
(Which, of course, they’re free to do)—
If, of the letters they take off
And sever the first three,
They leave the nine remaining
As sad as they can be:
For, though it seems to make it less,
In fact it makes it more,
For it takes the brute creation in,
Which it left out before.

Let’s try if we can mend it—

It’s possible we may,
If only we divide it
In some new-fashioned way,
Instead of three and nine, then,
Let’s make it four and eight;
You’ll say it makes no difference,
At least not very great:
But only see the consequence!
That’s all that needs be done
To change this mass of sadness
To unmitigated fun.
It clears off swords and pistols,
 Revolvers, bowie-knives,
And all the horrid weapons
By which men lose their lives;
It wakens holier feelings—
And how joyfully is heard
The native sound of gladness
Compressed into one word!

Yes! four and eight, my friends!

Let that be yours and mine,
Though all the hosts of demons
Rejoice in three and nine.

Answer

272.

A word by grammarians used in our tongue,

Of such a construction is seen,
That if, from five syllables one is removed,
No syllable then will remain.

Answer

273.

Formed long ago, yet made to-day,

I’m most in use when others sleep;
What few would like to give away,
And none would like to keep.

Answer

274.
 A lady was asked “What is Josh Billings’ real name? What do you
think of his writings?” How did she answer both questions by one
word?
Answer

275.
Why is Mr. Jones’ stock-farm, carried on by his boys, like the
focus of a burning-glass?
Answer

276.

A by <. The name of a book, and of its author.

Answer

277.
What word in the English language contains the six vowels in
alphabetical order?
Answer

278.
If the parlor fire needs replenishing, what hero of history could
you name in ordering a servant to attend to it?
 Answer

279.
My FIRST is an insect, my SECOND a quadruped, and my WHOLE
has no real existence.
Answer

280.
If the roof of the Tower of London should blow off, what two
names in English history would the uppermost rooms cry out?
Answer

281.

MY FIRST.

In the glance of the sun, when the wild birds sing,

I start in my beauty to gladden the spring;
I weep at the morning marriage, and smile
On the evening tomb, though I die the while.

MY SECOND.

I wander; I sin; though a breath may make

All my frame an effeminate nature take,
And a manly dignity that, as well,
Can of mastery and lordship tell.

MY WHOLE.
 I have startled the world to jeering and mirth,
Since that, earthly, I dared to withdraw from the earth;
But I stay, though cut off in my prime, far more
Enlivening and life-full than ever before.

Answer

282.

One hundred and one by fifty divide,

Then, if you add naught to the right or left side,
The result will be one out of nine—have you tried?

Answer

283.
I am composed of five letters. As I stand, I am a river in Virginia,
and a fraud. Beheaded, I am one of the sources of light and growth.
Beheaded again, I sustain life; again, and I am a preposition. Omit
my third, and I am a domestic animal in French, and the delight of
social intercourse in English. Transpose my first four, and I become
what may attack your head, if it is a weak one, in your efforts to find
me out.
Answer

284.

Unto a certain numeral one letter join—sad fate!

What first was solitary, you now annihilate.

Answer
 285.

My FIRST was heard to “hurtle in the sky,

When foes in conflict met in olden time”;
My SECOND none can yield without a sigh,
Though it has oft been forfeited by crime;
My WHOLE, its ancient uses gone, is found
On sunny uplands, or in forest ground.

Answer

286.

Can you tell me why

A hypocrite’s eye
Can better descry
Than you can, or I,
Upon how many toes
A pussy-cat goes?

Answer

287.

Walked on earth,
Talked on earth,
Boldly rebuked sin;
Never in Heaven,
Never in Hell,
Never can enter in.

Answer
 288.
There is a certain natural production that is neither animal,
vegetable, nor mineral; it exists from two to six feet from the surface
of the earth; it has neither length, breadth, nor substance; is neither
male nor female, though it is found between both; it is often
mentioned in the Old Testament, and strongly recommended in the
New; and it answers equally the purposes of fidelity and treachery.
Answer

289.

We are little airy creatures,

All of different voice and features:
One of us in glass is set;
One of us is found in jet;
One of us is set in tin;
One a lump of gold within:
If the last you should pursue,
It can never fly from you.

Answer

290.

My FIRST is a point, my SECOND a span;

In my WHOLE often ends the greatness of man.

Answer
 291.

Wherever English land

Touches the pebbly shore,
My FIRST lies on the sand,
Changing forevermore.
My SECOND oft, I’m told,
State secrets will hold fast,
But, to a key of gold
’Tis known to yield at last.
Fond mother, tender wife,
With agonizing soul,—
The exile, sick of life,—
Have looked and sighed my WHOLE.

Answer

292.

I begin with a thousand, I end with a hundred;

My middle’s a thousand again;
The third of all vowels, the ninth of all letters,
Take their place in the rest of the train:
My WHOLE is a thing you never should do,—
At least, you don’t like it, if tried upon you!

Answer

293.

A word which always speaks of shame

I pray you, reader, now to name:
Eleven parts my whole contains,
To guess them you must take some pains.
 Three groups there be which stand related;
The first with many a word is mated:
The second speaks of favor rare;
The third of plenty everywhere.

Cut off the first; and shameful grows

As fair as any garden rose;
Cut off the last, and lo! ’tis plain,
The word is full of shame again.

Answer

294.
The eldest of four brothers did a sound business; the second, a
smashing business; the third, a light business; and the youngest, the
most wicked business. What were they?
Answer

295.

Cut off my head, and singular I am,

Cut off my tail, and plural I appear;
Cut off both head and tail, O wondrous fact!
My middle part remains, though naught is there.

What is my head cut off? A sounding sea.

What is my tail cut off? A roaring river.
Far in the ocean’s depths I fearless play;
Giver of sweetest sounds, yet mute forever.

Answer

296.
I’m a creature most active, most useful, most known,
Of the thousands who daily perambulate town.
Take from me one letter, and still you will see
I’m the same as I was; just the same, to a T.
Take two letters from me, take three, or take four,
And still I remain just the same as before:
Indeed I may tell you, although you take all
You cannot destroy me, or change me at all.

Answer

297.

My FIRST is up at break of day,

And makes a welcome voice heard,
And goes to bed in twilight gray,
Though neither child nor song-bird.

My SECOND’S known to tongue and pen;

Is fast to all the church walls,
Is always seen in nurseries,
And often when the snow falls.

In green and yellow always dight,

Though melancholy never,
My WHOLE shines bright with golden light,
And emerald, forever.

Answer

298.

To fifty add nothing, then five,

Then add the first part of eighteen;
A desert would life be without it,
 But with it, a garden, I ween.

Answer

299.
What tree bears the most fruit for the Boston market?
Answer

300.
Why is the end of a dog’s tail, like the heart of a tree?
Answer

301.
Why is a fish-monger not likely to be generous?
Answer

302.
Take away my first five, and I am a tree. Take away my last five,
and I am a vegetable. Without my last three, I am an ornament. Cut
off my first and my last three, and I am a titled gentleman. From his
name cut off the last letter, and an organ of sense will remain.
Remove from this the last, and two parts of your head will be left.
Divide me into halves, and you find a fruit and an instrument of
correction. Entire, I can be obtained of any druggist.
 Answer

303.
Why was Elizabeth of England a more marvelous sovereign than
Napoleon?
Answer

304.
A SQUARE-OF-EVERY-WORD PUZZLE.
I.

The desert-king,
Whose presence will
Each living thing
With terror fill.

II.

Of this word ’tis the mission

To be a preposition,
Giving you a notion
Of onward, inward, motion.

III.

This charm to blend,

The myriad roses of Cashmere you ask
Their subtle essences to one small flask
Freely to lend.

IV.

The middy, to his labor trained,

The sun by sextant viewed;